Overdose and a Surprise for Cully

Last night someone at Detroit Children's Hospital made the mistake of giving Cully an overdose of ACTH, the drug we are so frantically trying to get out of Cully's system. He received a dose 5 times over what he should have gotten. Medical staff learned this late in the night and Cully was hooked up and tested again and again. The team has had close eyes on him but we are not seeing anything of the overdose at this time outside of an increase of cortisone and blood pressure. Because of this mistake we have lost the ability to continue the aggressive ween that began two days ago and now must increase his intake of ACTH and begin a ween that will take much longer. This is horrible for Cully.

Cully has not improved yet and he continues to hold fluid and is unable to do much of anything but lay on his back.We are not getting any better yet.

Today Cully has an amazing surprise and so did mum. My grandson whom we have not yet met. I surprised them by bringing lil Jaxon and my daughter to the hospital. I walked in with a handful of clothes for mum and acted as if I were about to drop them. Mum rushed over and lifted the clothes from my hand and found her beautiful grandson Jaxon in my arms. Cully's reaction would make a linebacker cry. We laid Jax down next to Cully and while he was unable to turn his head and look at him, his arm reached around him and he stroked Jaxon's hair, played beep the nose and held his tiny hand. The smile on Cully's swollen face was priceless and for a moment he was a very very happy boy.

Cully takes on a rare side effect of his drug

  At the time of this video three days ago we were concerned about our sons weight gain and had voiced our worries to the team at Detroit Children's Hospital several times. We were consoled and made to understand that the gains are all part of the ACTH drug which as we knew was a very scary drug but at the same time was also our chance to save Cully. And we had made a decision to try the treatment.  At one point we  took Cully to the local ER last week when we found pockets around his groin which looked like marbles under the skin. We were assured that these were pockets of fluid and they understood this drug would cause fluid retention. Home Cully went.

   Cully kept growing in size and weight by the day. He became unable to move, care to speak, and was more uncomfortable by the hour. His scrotum even grew to the size of a softball and we had enough. Julie and I decided it was time to make the call and get to Children's Hospital regardless of the conversations we had over the phone with them. Regardless of the knowledge of these doctors and researchers, we are the eyes and the parents. While it all seems wrong to think we know better than these doctors, well, sometimes we do.. After all, parenting in my book is held much higher a position that any CEO, government seat or yes, even a doctor. And I'm so glad we made that decision.

  Once at the ER the staff took one look at Cully and made him a priority. He was admitted and during the two hours since we had left home Cully had grown in size and weight. His scrotum now so large he could not sit or move and he was in horrible pain. He was completely inconsolable and began screaming, which would not end for several hours. Eyes were unable to open fully as his face was so swollen, his weight now was 62 pounds. Just 19 days earlier Cully was 36 pounds.

  Nobody had a clear understanding just how bad Cully's health was. The staff speaking with us on the phone thought we were expressing the same concerns most parents have while on the ACTH drug. Yes, the cheeks swell, the weigh increases and they become uncomfortable. We were very concerned but at the same time we felt  that the staff did a good job convincing us that what we were seeing was the norm. It took the hospital to actually see Cully for everyone to understand that what was happening was NOT NORMAL and Cully was in serious danger.

 Cully and mum remain in the hospital. Tests after tests only tell us that Cully's kidneys have failed. The extent of their damage or if the damage can be reversed is still unknown. It seems likely that the ACTH and or the several other drugs he is on has done this. The ACTH can not simply be quit. It is a weening process that we had already began but the neurology teem took no time to make an unheard of reduction in the ACTH straight away. His ween will be a very aggressive one now.

  The first issue at hand now is to rid Cully of all of this fluid trapped in his body. The difficult part is that the tools used to remove fluid removes potassium as well. His potassium has crashed very low again. In as much time as he has been in the hospital Cully has lost 4 pounds. He has a long way to go yet. His scrotum is still painfully large and he can not move or sit. His life at this time is laying in the crib and having one test after another.. Yet he still manages to smile and be his usual gentleman like self. The screams have stopped.

  He is being observed by several teams from many departments and at record visits per day. Cully has become the hospital favorite once again and staff from all floors continue to visit him just to say hello. But we do not have all the answers yet. We have no idea what to expect in regards to his kidneys and all we can do is hope that they can be saved. He has a long road ahead of him and with so much unknown at this point we have little comfort in what the outcome is.

 The wonderful news in all of this is that Cully is still 100% seizure free.  While that may sound like a small accomplishment in comparison to Cully's current situation I can not find the words to express just how incorrect that would be. These seizures that Cully was having, the infantile spasms, were taking Cully from us. There was very little chance that we could stop the seizures but we chose to try these treatments just the same so that he had a chance. The alternative was not good. As the medical definition states... "Infantile Spasms is one of the most catastrophic forms of epilepsy". We are very lucky in this respect. We are winning that battle. We were on the down hill side when all these new problems came about and we need to get past all this so Sawyer can teach Cully to ride bikes.....  We just need to hear the kidneys will come back.

Far too much going on, too little time

Cully is again at Children's Hospital. Holding his own though. Will post more information soon.

Introducing... Mum

Since the only update I can give is  "ALL GOING WELL"....  I figured this would be a good time to intro the good looking gal that everyone here calls MOM......
  This is my "Jewels" and she is the reason we all keep our sanity.... Thanks for everything honey...

Introducing "UNCLE CULLY"

Cully Gage became "Uncle Cully"

Jaxon born 6 lbs 15 0z at 2:49 pm, November 13th, 2012

WE ARE WINNING !!!!! This is huge!

Cully's original EEG on November 6th turned into a near week long stay at Children's Hospital when his health took a turn for the worse.  After his discharge Cully had another bout of the muscle spasms in his arm which was linked to the low electrolyte levels so he returned to the hospital for a day stay and an IV.

NOW THE GOOD NEWS !!

We returned again for another EEG yesterday as a follow up after the increase in ACTH. the results were GREAT !  Not a single sign of any seizure activity in his brain. Nor had we seen a visible seizure all week. The ACTH has done it... And now we start to wein him off the ACTH which will take 5 weeks.  We were informed that there are some children that may need a second round of the ACTH and we will see how this goes over the next couple months.  BUT IT WORKED !!

We knew something was up. Cully has been doing so good. The last few days he has been putting words together to make a full sentence. His speech is a little clearer too. His attitude has improved too. Without all that erratic activity in his brain he has been able to focus and stay on task.


ANOTHER BUMP THOUGH....

Today Cully had to return to the hospital once again due to the muscle spasms. They were much worse than we had ever seen. This time not in his arm but in his legs. Starting in the left and working to the right. He came yelling OWE..STOP IT...HELP..... The legs were twitching and cramping and it hurt him a lot. I think it could be described as the feeling of a charley horse that goes on for a while then to stop for a moment and return again. He was crying and begging for help but there was nothing we could do but panic, which we did. He would walk for a moment and then both legs would give out and he would fall to the floor and grab his legs screaming. This happened a few times while we ran around getting things for the  trip to the hospital. To see it was really frightening as you could not miss the legs shaking so fast and when I would hold his legs you could feel the muscles inside pull and release like cables under his skin. Imagine being a 2 year old trying to understand this.

Blood drawn and tests came back and showed that Cully's levels are all normal or better.  So now the issue is to determine what is causing his muscles to do this and how can we get him immediate relief? Is this due to the ACTH treatment or something else?

Cully has gained 11 pounds from the ACTH in less than two weeks. I think half of it is in his cheeks...

STOP THE PRESS!

In a strange turn of events, which seems to be rather common lately regarding Cully.....

CULLY IS HOME !!!     CULLY IS HOME!!!

Figuring that he would be more comfortable at home and be closely monitored, Cully was sent home this evening.  His potassium level was good this afternoon, calcium too. So home he went. Thursday back to Children's Hospital for tests and nothing but family time at home til then. Lots and lots of family time.. :>)

Cully went in to the hospital at 36 lbs.  He returned home a whopping 42 lbs this evening.  The ACTH is REALLY DOING ITS JOB !  

NO SEIZURES AT ALL ....................................

Roller coaster days

Just when you think you have things figured out...  Uhgh. ..

This morning Cully's potassium crashed very low again, he has a cough and the sniffles, and his blood pressure is crazy too.

Just going to have to take it day by day but he will not be on his way home any time soon. Cully's Mum has not left his side all week. I think they are enjoying all the time they get to be together.

Just another little bump in the road..

Cully had a visitor by a therapy dog.  He loves puppies. Even if they are as big as a horse.

What a great day

Lots of smiles all around. Cully has perked right back up and is interacting almost like usual.

AND NO SEIZURES AT ALL!!

 Today his big sister got to surprise him with a visit. They walked together, played and Elora even fed Cully a nice supper.  The left arm has completely stopped shaking, he digestive system has improved a lot, and the potassium level reached almost normal before declining a bit again.

 So here is what seems to be happening. The ACTH drug is causing the kidneys to not allow the potassium, electrolyte  or calcium levels to remain normal. But...  the kidneys have not failed. They simply are tricked into not building these levels in Cully while the ACTH is present. No damage to the kidneys has been done and once the treatment of ACTH is complete the kidneys will function the way they should.  Until then we will be giving Cully supplements of all three and he will be monitored very closely.  The ACTH was also increased in the middle of all of this...... And he has not had a seizure.

 Low potassium will cause the digestive system to not do its job. Replenish the potassium and things should function again. Low electrolytes will cause muscle spasms. Replenish those and the muscle spasms/ shaky arm should stop.  It has...

 Further tests tomorrow should tell us if this is all correct or if we have something else going on. If things do stabilize in his levels and Cully's digestive system comes back to par then he may even come home very soon.  His tummy still continues to get very hard he is having a good bit of pain with cramps and such. Things just have not come back enough yet in that department so fingers are crossed that we get this worked out. Another thing we need to monitor is his heart. He has had some episodes of low heart rate and palpitations.

 We were in such bad shape over these past few days and now we see things looking so cheery. We have gone from desperation to excitement. My boys have really shined with all of this. Sawyer has been so understanding and patient. Doug has been so grown up and helpful, kept to his school work and been kind with his words and frustrations. He has been a true adult through this and I think these experiences are shaping his future.

  I think now I can relax and breath.....

Adding a twist

I need to put together a better post. Something made up of more than sad depressing news.  I'm going to toss one out tonight with a touch of fact be it good or bad, a twist of smiles, and a dash of giggles.

I have a problem with keeping my chin up and seeing the positive at each turn... Just call me Eeyore.. I never forget to find the good but it takes me a bit longer than most when I get overwhelmed. And I get overwhelmed often.. But here you go... Cully >>>>

  Cully just can not seem to get his potassium level up regardless of what the team at Children's Hospital does. And the team has told us they think they may have an answer..  It looks like it is Cully's kidneys. And the ACTH may be a contributor. The ACTH treatment is continuing at this point but we do not know what will come tomorrow. His calcium levels are low as well and the "Kidney team?" visited and explained that it is a sign of kidney problems.  So we wait to learn what to do about that....

  Digestive system has not been moving but we have signs that it is getting back to work now. We hope to see even better signs tomorrow..

  Cully is beginning to snap out of his TEENAGER stage where all he does is lay in bed all day and whine. After sleeping the whole week away we found Cully playing with his brothers for a few minutes last night. This evening he and I took a stroll around the 6th floor in his stroller with Cully wearing a mask. We even made a pit stop at the play room and he had a few minutes of fun. He even stood up on his own two feet and walked a bit. So while he is still spending most of his time unconscious in his bed he is also showing better signs of alertness and playfulness. 

  Cully grew. He is HUGE and this is just in comparison to yesterday. It doesn't even seem real but he is too much to lift, to big to wear any of his close and he is strong as an ox be it sick or healthy.  Eyes are somewhat swollen closed but nothing uncomfortable for him. This is most likely his medication.

  This morning we gained one more hurdle. Cully's left arm is shaking from time to time. The neuro team checked him out and it is not a seizure. It does come and go but boy does this get Cully MAD! I handed him the Ipad and he could not get his arm to function so he began yelling at it "NO NO STOP IT"  The crew is working on that one as well and this could be something as simple as a muscle spasm due to his low levels or his medications.

 I had a nice evening with him while he sat on my lap for a moment and the little strolls we took, the singing to a Barney video and he even gladly accepted a backrub from me. When I went to leave tonight he was laying on his belly and he kept shuffling his huge self to the side. We had no idea what he was doing but in the end he got his face up against the bars of the crib and pushed his mammoth chubby cheek through so I could give him a kiss goodbye. That took a lot of work (and a lot of time) to move that large of a load across the bed just for a kiss and it made it all the more enjoyable.

  Now..... We have had several people treat us so well these past days. Emails, calls and texts. Even a couple emotional posts on Facebook in our favor. Julie and I can not clearly explain what it means to us to have people, friends  be there for us like this. We have needed it too. Especially me as I get in my crazy state. Thank you for being there with the offers of help, the checking in on us, and keeping Cully in your thoughts. We are learning what true friends are all about.  

 Things are improving in ways and the more we learn the closer we will be to making things right. I would place my bet on Cully returning home soon in much better health and making us all laugh with his goofy acts.

  On another note... Tonight I made a stop on the way home and picked up Cully's big sister Elora. Now when I say big sister I mean BIG SISTER.. Elora is due to make me a Grandpa any minute. YES ! GRANDPA !.. Now I don't know much about this grandpa thing but I think it means that I get a discount on my coffee, am supposed to leave my turn signal on at all times to announce that I belong to the *Grandpa Club* and that I now have a responsibility to double check each and every one of my receipts and contest them when incorrect by even as much as 1 penny.   Why are you doing this to me Elora???  I'm glad you are here..
 

another update

The doctors made another visit this evening. Cully has received four IV bags of potassium and his levels have not increased and remain dangerously low. He also has thrush and is now receiving an antibiotic.

They seem puzzled as to what is going on.

Update to Cully Gage

 When I left the hospital last night to come home to Doug and Sawyer we were uncertain what we would be expecting with Cully. He had a rough evening with tears and screams. He was given a large amount of potassium over night and his tummy improved and his potassium level raised. In the morning even after the flood of potassium his levels dropped dangerously low again. The ACTH had been increased. Cully had settled down and did go back to a solid sleep that continued through much of today. He continued to not want to be touched. His potassium intake is now increased to a tremendously large volume and we will wait to see if the levels rise and hold..

   Dougie and Sawyer visited today and after a little while I found Cully sitting up and laughing hysterically while they all played together. THIS WAS HUGE !  and only Dougie and Sawyer could pull this off...

The doctors seem to think that there is a chance the ACTH may be causing the potassium levels to drop. They feel taking Cully off the ACTH is unacceptable as we need to try to stop the seizures. If they determine the  potassium levels to be due to the ACTH then they will give Cully a supplement for his potassium and continue the treatment. Levels are not increasing yet so they are going to continue to monitor him closely and figure this all out.  

  The ACTH drug will keep Cully's body from having a temperature until infections are deathly over running his body. We just can not tell if he is sick or if he is responding to reactions from his medications. Cully's medication list at this time.

Onfi

Vigabatrin

ACTH

Multivitamin

B6

Zantac

Omega 3 DHA

Taurine

Vitamin C

Melatonin

Potassium

Cully is OK right now.

Cullys test cut short

Cully's EEG was cut short today. He was taken down to emergency not doing well at all. His potassium level  has been declining and hit a very low level. His tummy is tight as a drum and he was acting lethargic. I put my finger in his hand and he cried and pulled away. Seems to be achy and have a headache too. Cully has been sleeping all day too which is very unlike him.

  Radiology took two sets of Xrays and questioned if he had any sort of a radioactive fluid test done recently??  No never!!  they say that Cully may have had eaten something then as there is something showing up like that in the Xrays.  They then said it could be some sort of lead that he ingested??   See earlier posts where I mention that Cully eats EVERYTHING.. Pika.  It is possible but what he ate we have no idea.

 Once admitted to the hospital he was transferred upstairs and is being taken care of by his mummy. Julie just called and Cully is screaming non stop. He is just miserable tonight.  He has an IV drip of potassium and ?? and is being watched.

  The wonderful Dr Chugani held his meeting with us in the emergency room. Cully's short EEG showed plenty of seizures and hypsarrhythmia. Definition? Hypsarrhythmia is a high voltage disorganized pattern of EEG abnormality in the brain. Basically the IS.

  The doctor has recomended we increase the ACTH and keep trying as the alternative is surgery and he does not even want to discuss that yet. He also explained that Cully is having secondary seizures called "Tonic seizures".

  I still was uncertain of the type of diagnoses of IS that was given. It takes me a while to understand all of this so I had to come right out and ask him. I see  the other families mentioning the complete diagnosis of their child and I could not explain our situation. All of this still seems foreign to me and I can not put the pieces together.   Dr Chugani explained that we have a Symptomatic diagnoses of IS which means we have found a cause. Cully has a focus in his front temporal lobe and it appears to be a single focus. This focus is located in a good area in regards to the surgery/removal if we should go that route. He feels that some of the things we are seeing with him such as the sensory disorder and aggressive non stop behavior could be linked to this focus as well.

  My son has several positive things going for him while in a rather unfortunate situation. Now we just need to get him better so we can keep moving on with the treatment. 

Correction... Flu

Cully has the flu. Flu and ACTH does not make a good combination. Took him in, doing good and will still have the EEG and such in the morning.

One step closer to overload. It's the non stop snuggles I keep getting from Cully out of the blue that keeps me grounded.   Does anyone else find it odd that the little boy with autism seems to show more social care and close interaction than most children do?

... So help me Cully ! If you're faking it !          well.. I'll be pretty happy...

Starting to look grim ** but no Flu

Sawyer is better and turned out he only had a sour tummy and no flu.

  Cully is still having seizures while on the ACTH for almost two weeks.  This morning he had the worst seizure yet and it caused him to fall down. This is about the worst thing we could see right now.

 Outside of the seizures he seems ok. The tempers and the whiny attitudes are as expected so not worried about that. He loves to dance and interact with everyone. He is smart and still is learning a lot of things.

  Something I am noticing a lot of however is he is mumbling almost everything. Not quietly but rather just all mushed together where we can not make out a lot of what he is saying. Maybe this is part of the drug or could this be his regression? He is not hesitating to talk though. Every time we hand him something or he asks for something it comes with a "Please" or "Thank you"  and he is ALWAYS wanting something. But our first regression at the start of all this was his speech so there is a sign that this is not the drug unfortunately. Fingers are crossed...

  Our days are still filled with snuggles where Cully will get next to us and hug us or just lay on us. He has his sense of humor which is very prominent every day with tickling us, laughing and hiding around corners to scare us with a "BOO".

  The patients project is all but out the window. Cully gets very frustrated with the Ipad now and has no tolerance for much of anything that does not work just right. This he gets from his Daddy though *sigh*.. Guilty as charged....

    Tuesday we return to the hospital for the EEG and we also meet with the doctor to see what to do next..